8824/0 Myofibroma


Definitions

Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49

Myofibroma (or myofibromatosis in the case of multicentric lesions) is a benign condition arising within soft tissue or bone, histologically characterized by contractile myoid cells arranged around thin-walled blood vessels, with a nodular or multinodular growth pattern. Plump, spindle shaped myofibroblasts are generally located in the periphery of the lesion and arranged in short fascicles or whorls, while less differentiated cells predominate in the centre of the nodules 1
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Myofibroma of soft tissue affects the cutaneous/subcutaneous tissues of head and neck, trunk, and extremities. Myofibromatosis also arises in the deep soft tissues and at visceral locations. Lesions in dermis and subcutaneous tissue are generally better defined than those in the deep soft tissues and viscera.
Myofibroma/myofibromatosis occurs in all age groups with a predilection for males. Myofibromas may regress spontaneously. Recurrence after excision is rare. The prognosis for visceral lesions depends on extent and location
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Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Skin
ICD-O-3 topography code: C44

Dermatomyofibroma is a distinct biologically benign fibroblastic/myofibroblastic cutaneous proliferation occurring predominantly in young female patients.